新加坡系统性硬化症队列中间质性肺病进展的预测因素:一项多中心研究。

Maria Noviani, Seyed Ehsan Saffari, Gim Gee Teng, Xin Rong Lim, Grace Yin Lai Chan, Amelia Santosa, Cassandra Hong, Sue-Ann Ng, Andrea Hsiu Ling Low
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引用次数: 0

摘要

导言:系统性硬化症(SSc)患者的间质性肺病(ILD)具有异质性,其进展速度各不相同。本研究旨在确定在确诊 ILD 后 1、3 和 5 年内与 ILD 进展相关的基线临床特征:这是一项前瞻性多中心研究--新加坡系统性硬化队列研究--于2008年1月至2021年2月进行,研究对象包括经高分辨率计算机断层扫描确诊患有ILD的SSc患者。强迫生命容量(FVC)下降≥10%(预测值)或FVC下降5%-9%(预测值),一氧化碳肺弥散容量下降≥15%(诊断为ILD时),即为ILD进展。在对恶性肿瘤和治疗进行调整后,进行了多变量逻辑分析和 Cox 回归分析,以确定 ILD 进展的独立风险因素:在124名患有ILD的SSc患者中,47.6%患有局限性皮肤SSc,33.9%患有弥漫性SSc,18.5%患有SSc-重叠性SSc。分别有6%、15%和23%的患者在1年、3年和5年内出现ILD进展。调整恶性肿瘤和治疗因素后,抗La与1年内(几率比[OR]6.94,95% 置信区间[CI]:1.14-42.2;P = 0.04)和3年内(OR 5.98,95% CI:1.31-27.4;P = 0.02)的ILD进展相关,抗Scl-70与5年内的ILD进展相关(OR 2.54,95% CI:1.05-6.12;P = 0.04)。将 ILD 进展时间作为一项结果进行分析,抗-La 与较高的 ILD 进展风险显著相关(危险比 3.47,95% CI:1.18-10.2;P = 0.02)。抗La患者的ILD进展时间为1.4年,而无抗La患者为6.9年(P = 0.02);抗Scl-70患者的ILD进展时间为4.7年,而无抗Scl-70患者为8.9年(P = 0.12):结论:在这批亚洲 SSc 患者中,自身抗体有助于预测 ILD 的进展率。
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Predictive factors for interstitial lung disease progression in a Singapore systemic sclerosis cohort: a multicentre study.

Introduction: Interstitial lung disease (ILD) in systemic sclerosis (SSc) is heterogeneous with varied progression rate. This study aimed to identify the baseline clinical characteristics associated with ILD progression within 1, 3 and 5 years of the diagnosis of ILD.

Methods: This was a prospective, multicentre study - Systematic Sclerosis Cohort Singapore - conducted from January 2008 to February 2021, which included SSc patients with ILD diagnosed by high-resolution computed tomography. Progression of ILD was defined by forced vital capacity (FVC) decline ≥10% predicted or FVC decline 5%-9% predicted, with diffusing lung capacity of carbon monoxide decline ≥15% from the time of ILD diagnosis. Multivariable logistic and Cox regression analyses, adjusting for malignancy and treatment, were performed to determine independent risk factors of ILD progression.

Results: Of 124 SSc patients with ILD, 47.6% had limited cutaneous SSc, 33.9% had diffuse SSc and 18.5% had SSc-overlap. Progression of ILD was seen in 6%, 15% and 23% of patients within 1, 3 and 5 years, respectively. After adjusting for malignancy and treatment, anti-La was associated with ILD progression within 1 year (odds ratio [OR] 6.94, 95% confidence interval [CI]: 1.14-42.2; P = 0.04) and 3 years (OR 5.98, 95% CI: 1.31-27.4; P = 0.02), and anti-Scl-70 was associated with ILD progression within 5 years (OR 2.54, 95% CI: 1.05-6.12; P = 0.04). Analysing time to ILD progression as an outcome, anti-La was significantly associated with higher risk of ILD progression (hazard ratio 3.47, 95% CI: 1.18-10.2; P = 0.02). Time to ILD progression was 1.4 years in patients with anti-La versus 6.9 years in patients without anti-La (P = 0.02), and 4.7 years in patients with anti-Scl-70 versus 8.9 years in patients without anti-Scl-70 (P = 0.12).

Conclusion: In this Asian cohort of SSc patients, autoantibodies may help to predict ILD progression rates.

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